From: Long-term clinical course and outcomes in patients with lymphangioleiomyomatosis
Characteristics | Total | Non-survivors | Survivors | P-value |
---|---|---|---|---|
Number of patients | 104 | 9 | 95 | |
Pneumothorax | 3 (2.9) | 0 (0.0) | 3 (3.2) | > 0.999 |
Chylothorax | 2 (1.9) | 0 (0.0) | 2 (2.1) | > 0.999 |
Extrapulmonary manifestations | 3 (2.9) | 0 (0.0) | 3 (3.2) | > 0.999 |
Angiomyolipoma | 3 (2.9) | 0 (0.0) | 3 (3.2) | > 0.999 |
Lymphangioleiomyoma | 0 (0.0) | 0 (0.0) | 0 (0.0) | > 0.999 |
Disease progression | 33/92 (35.9) | 4/7 (57.1) | 29/85 (34.1) | 0.245 |
Lung function decline | N = 92 | N = 7 | N = 85 | |
FEV1, % predicted/year | − 0.7 ± 4.2 | − 2.0 ± 5.4 | − 0.7 ± 4.1 | 0.448 |
FVC, % predicted/year | 0.3 ± 3.2 | − 2.2 ± 4.3 | 0.8 ± 3.1 | 0.147 |
DLCO, % predicted/year | 1.5 ± 4.0 | − 1.1 ± 3.6 | − 1.5 ± 4.1 | 0.929 |
RV, % predicted/year | − 1.0 ± 9.1 | 2.7 ± 4.9 | − 1.3 ± 9.3 | 0.221 |
FEF25–75, % predicted/year | − 2.4 ± 7.4 | 1.6 ± 8.8 | − 2.8 ± 7.2 | 0.185 |
Follow-up period, year | 7.1 (2.8–9.9) | 2.7 (2.5–9.6) | 7.2 (3.0–9.9) | 0.236 |